In APL, what markers are lost? Aberrantly expressed? Which aberrant markers have prognostic implications?
CD15 (normal promyelocyte is CD15+/-); CD2 (microgranular variant), CD64 and CD56; CD56 expression poor prognosis
What marker is characteristically expressed in megakaryocytes/megakaryoblasts in AML M7 (megakaryoblastic) and MDS?
CD34 (usually CD34+/- in megakaryoblasts)
What is required for a diagnosis of MPN/MDS?
Features of MDS (fits a category of MDS w/dysplasia and <20% blasts) + MPN (leukoerythroblastosis with proliferation of at least one lineage) + NO characteristic cytogenetic abnmlty (BCR-ABL, PDGFR, FGFR1, etc)
Which APL translocations are not sensitive to Trans-retinoic acid tx?
t(11;17)(q23;q11) with PLZF-RARα and t(5;17), also short bcr3 breakpoint which has features of M3v
What causes inclusions/phagocytosis in pts with APL? What else could cause this finding?
Trans-retinoic acid tx; possibly GCSF, which could push myelos to maturation in APL (could kill pt?)
What mutation is often seen in histiocytic malignancies?
BRAF
What is the characteristic feature of anemia in RA-RS?
Normo or microcytic anemia
What is a negative prognostic indicator in inv16 AML (eosinophils)?
Kit mutation (also negative impact on incidence of relapse but not overall survival in t(8;21)
CD4+, CD56+, CD123+, TCL1+, CD33+, CD68+, sometimes CD7+ and TdT+
What is the most common cytogenetic abnormality in T-prolymphocytic leukemia? Other abnormalities?
Inversion of chromosome 14 with breakpoints in q11 and q32; 10% t(14;14)(q11;q32); chromosome 8 abnormalities: trisomy 8q or idic 8p11, or t(8;8)
What T-cell lymphoma has isochromosome 7q in most cases?
Hepatosplenic T-cell lymphoma
What is the prognosis for the following cytogenetic abnormalities in AML? How would a kit mutation affect prognosis?
A) inv16
B) t(8;21)
C) t(15;17)
D) t(16;16)
All good prognosis; kit mutation associated with a poorer prognosis/early relapse
What is the prognosis for the following cytogenetic abnormalities:
A) monosomy 7
B) inv(3)
C) 11q23 abnormalities (except 9;11)
D) t(9;11)
E) trisomy 8
F) normal cytogenetics
A) monosomy 7 - poor
B) inv(3) - poor
C) 11q23 abnormalities - poor
D) t(9;11) - intermediate
E) trisomy 8 - intermediate
F) normal cytogenetics - intermediate
All of the following cytogenetic abnormalities are not considered definitive evidence of MDS except:
B)
Which of the following CLL genomic aberrations (more than one may apply) have a less favorable or poor prognosis:
A) del(11q22-23)
B) del(6q)
C) isolated del(13q14.3)
D) del(17p)
E) Trisomy 12
All except C
All of the following are chemotherapy agents involved in therapy related MDS/AML except:
Which agent usually doesn't have an MDS phase?
A) Topoisomerase inhibitors
B) Alkylating agents (cyclophosphamide, nitrogen mustard, cisplatin, carboplatin)
C) Ionizing radiation therapy
D) Antimetabolites (5-FU, 6-MP, Ara-C)
E) Antitubulin agents (vincristine, docetaxel)
D; A - topoisomerase inhibitors do not have an MDS phase
In AML t(8;21), what are the genes? What do they encode?
Chrom 8 - ETO gene; chrom 21 is AML1 gene, which encodes the alpha chain of core binding factor (CBFalpha)
What is the staining pattern for AML M0 with respect to sudan black (SBB), MPO, CAE, and NSE? Are Auer rods seen?
Negative for all; Auer rods are rare
What do SBB and CAE stain for?
SBB - myeloids and erythroids (but negative in AML M0 and M5 (monoblasts) and M7 (megas)
CAE - granulocytes (but negative in AML M0 and M1)
In ALL with t(4;11), what will you characteristically find in the immunophenotypic profile?
CD15+/CD10-
What is the common protein size for ALL with t(9;22)?
190kD
Variant translocations in PML? Treatment implications? What breakpoint is associated with the variant M3 (M3v)?
t(11;17) and t(5;17); resistant to ATRA; short breakpoint (bcr3, intron 3)
Common sites for extramedullary plasmacytoma? Plasmacytomas in bone? Do they develop MM?
Nasal cavity, oropharynx, larynx; vertebrae > ribs > pelvis; 75% of bone plasmacytomas develop MM within 10 yrs; extramedullary plasmacytomas do not develop MM
Diagnostic criteria for smoldering myeloma? MGUS?
Smoldering: 10-30% plasma cells, no CRAB
MGUS: <3.5g/dL gammopathy, <10% plasma cells, no CRAB
Most common form of heavy chain disease?
Alpha heavy chain
Where is the BCL6 gene located? Common translocation in DLBCL?
Chrom 3q27; t(3;14) involving heavy chain
What lymphomas have t(14;18)
90% follicular lymphoma, small minority in DLBCL; rare in other B-cell lymphomas
Translocations in extranodal MZL?
1) t(11;18) involving API2 and MALT1 genes, usually in stomach and lung
2) t(14;18) involving MALT1-IgH gene fusion, usually in ocular, parotid, cutaneous sites
What is the expression of cyclin D1 in hairy cell leukemia?
Dim positive
Pathognomonic finding in plasma cells of heavy chain disease?
Vacuolated cytoplasm
All of the following are minor criteria for primary myelofibrosis except:
D)
T/F: Essential thrombocythemia demonstrates BM with increased granulocytes with left-shift.
False - only PV and PMF have this
How can the vacuolated erythroid precursors in MDS be differentiated from other causes (ETOH, CDA, anti-seizure drugs)?
Vacuoles in MDS are poorly defined and sharply delineated in toxic alteration
In pediatric follicular lymphoma, how does it differ from adult form?
1) BCL2 negative, 2) usually grade 3 but good prognosis, 3) no t(14;18)
How does cutaneous follicle center lymphoma differ from nodal FL?
Usually BCL2 negative and no BCL2 rearrangements, CD10 positive only in follicular patterns not diffuse, overall good prognosis
What virus is associated with PTLD? How does the polymorphic differ from monomorphic type?